A 39-year-old woman, who 3 months prior had undergone sono-guided biopsy for pelvic masses, was admitted to our hospital with dyspnea. After the procedure, a large arteriovenous (AV) fistula was detected. The patient developed dyspnea with pain and swelling of the right leg and subsequently visited our hospital. Multiple cafe-au-lait spots and subcutaneous nodules were noted and a continuous bruit was heard in the right pelvic area. Transthoracic echocardiography revealed marked dilatation of the right atrium, right ventricle, and inferior vena cava. An angiogram showed multiple AV fistulas between the right iliac arteries and veins. The patient underwent aortoiliofemoral bypass surgery. Follow-up echocardiography after surgery showed dramatic improvement in heart function. Molecular analysis revealed a novel variation (c.4270-1G>a) at the 24th intron-exon splicing consensus sequence of the neurofibromin 1 (NF1) gene. This is a rare case of AV fistula that developed after biopsy and caused right-sided heart failure in a patient with neurofibromatosis.
Adult ; Arteriovenous Fistula ; Biopsy ; Cafe-au-Lait Spots ; Consensus Sequence ; Dilatation ; Dyspnea ; Echocardiography ; Estrogens, Conjugated (USP) ; Female ; Fistula ; Follow-Up Studies ; Heart ; Heart Atria ; Heart Failure ; Heart Ventricles ; Humans ; Iliac Artery ; Leg ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibromin 1 ; Veins ; Vena Cava, Inferior

BACKGROUND: Non-invasive positive pressure ventilation (NPPV) ensures adequate gas exchange during bronchoscopy in spontaneously breathing, hypoxemic patients, thus avoiding endotracheal intubation. However, in some patients, endotracheal intubation is eventually required after bronchoscopy. This study investigated the incidence of intubation and predictors of a need for emergency intubation prior to NPPV bronchoscopy initiation. METHODS: On a retrospective basis, we reviewed the medical records of 36 patients (median age, 55 years; interquartile range [IQR], 43~65 years) with acute hypoxemic respiratory failure who required NPPV during bronchoscopy between January 2005 and October 2007. RESULTS: All patients were hypoxemic (median PaO2/FiO2 ratio 155; IQR 90~190), but tolerated bronchoscopy with NPPV support. SOFA score and SAPS II score immediately before NPPV initiation were 4 (3~7) and 36 (30~42), respectively. Seventeen (47%) patients needed endotracheal intubation at a median time of 22 (2~50) hours after bronchoscopy. Patients who needed intubation after bronchoscopy had a higher in-hospital mortality (11 [65%] vs. 4 [21%], p=0.017). Upon multiple logistic regression analysis, the need for intubation after bronchoscopy was independently associated with a PaO2/FiO2 ratio (OR, 0.961; 95% CI, 0.924~0.999; p=0.047) immediately before NPPV initiation for bronchoscopy. CONCLUSION: The severity of the hypoxemia immediately prior to NPPV initiation for bronchoscopy was associated with the need for intubation after bronchoscopy in patients with hypoxemic respiratory failure.
Anoxia ; Bronchoscopy ; Emergencies ; Hospital Mortality ; Humans ; Incidence ; Intubation ; Intubation, Intratracheal ; Logistic Models ; Medical Records ; Positive-Pressure Respiration ; Respiration ; Respiratory Insufficiency ; Retrospective Studies

We report a case of adult-onset tubulointerstitial nephritis and uveitis syndrome with Fanconi syndrome. A 31-year-woman presented with fever, anorexia, nausea, general weakness and weight loss for two months. Her initial laboratory findings showed anemia, high serum creatinine, hypouricemia, hypophosphatemia, hypokalemia, glucosuria, and proteinuria. She was diagnosed as having acute tubulointerstitial nephritis by renal biopsy. The etiology of tubulointerstitial nephritis was unclear. She was treated with systemic corticosteroid. Six months later and while the patient was still on systemic corticosteroid (Deflazacort 36 mg), bilateral uveitis developed. Renal function was recovered by systemic corticosteroid and mycophenolic acid. But ocular symptoms relapsed twice despite systemic corticosteroid treatment. The ocular symptoms improved after topical ophthalmic steroid drops and injection. Tubulointerstitial nephritis and uveitis syndrome should be considered in the differential diagnosis of the unexplained tubulointerstitial nephritis. And the need of the steroid treatment also should be considered in the case of adult-onset.
Adult ; Anemia ; Anorexia ; Biopsy ; Creatinine ; Diagnosis, Differential ; Fanconi Syndrome ; Fever ; Humans ; Hypokalemia ; Hypophosphatemia ; Korea ; Mycophenolic Acid ; Nausea ; Nephritis, Interstitial ; Proteinuria ; Steroids ; Uveitis ; Weight Loss